Cushing's Syndrome in Childhood: Report of Two Cases with Bilateral Adrenocortical Hyperplasia, Showing Distinctive Clinical Features

Abstract

Two cases of Cushing's syndrome resulting from adrenocortical hyperplasia arising in childhood are described. The distinctive features of these children were: 1) They were totally asymptomatic except for marked obesity associated with complete cessation of growth, resulting in stunted stature, and 2) they had submissive personalities and exhibited compulsive diligence in their schoolwork, which were no longer evident after adrenalectomy. Despite the patients' paucity of symptoms, the diagnosis was strongly suggested by the presence of characteristic physical findings and readily substantiated by the demonstration of the classic derangement of steroid excretion. Treatment consisted of total bilateral adrenalectomy. In each instance an entirely satisfactory result was obtained, with reversal of the biochemical and clinical abnormalities. In view of the numerous practical and theoretical disadvantages of the alternative modes of therapy, bilateral adrenalectomy appears to be the treatment of choice at present. It seems likely that Cushing's syndrome due to bilateral adrenocortical hyperplasia in childhood is commoner than the rarity of published reports would suggest, and that lack of the symptoms characteristic of the syndrome in adults may result in this disorder being overlooked in childhood.